Further Outpatient Care

Failure to thrive is common, and, in some studies, more than 50% of patients are below the 25th percentile for height and weight during the first year of life. In one study, one third of infants required gastrostomy tube placement to improve caloric intake. The need for supplemental oxygen at the time of discharge is a significant predictor for subsequent growth failure. Possible causes include increased caloric requirements due to chronic lung disease, oral aversion after prolonged intubation, poor oral feeding due to neurologic delays, and gastroesophageal reflux.

Because of the risk for CNS insult and sensorineural hearing loss, infants should be closely monitored for the first 3 years of life,
[19] preferably in a specialty follow-up clinic. These risks are particularly high in infants who are discharged home on supplemental oxygen. Reassess hearing at age 6 months (and later if indicated) because late sensorineural hearing loss occurs in approximately 40% of patients.

Even if a child has no major neurodevelopmental delays, he or she should be evaluated prior to entering school to determine if any subtle deficits may predispose the child to learning disabilities.

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Further Inpatient Care

Pulmonary care in congenital diaphragmatic hernia (CDH)

Severely affected infants have chronic lung disease. These infants may require prolonged therapy with supplemental oxygen and diuretics, an approach similar to that for bronchopulmonary dysplasia. The use of steroids, particularly high doses for prolonged periods, is controversial and may hinder appropriate lung and brain development.

Late pulmonary hypertension has been successfully treated with low-dose inhaled nitric oxide. This therapy can be delivered via nasal cannula following extubation. In this setting, the delivered dose is diluted because of entrainment of room air. In a recent report, the median duration of treatment using inhaled nitric oxide delivered via nasal cannula was 17 days.

Case reports are now emerging regarding the use of other pulmonary vasodilators, such as sildenafil and endothelin receptor antagonists. Systemic study is required to assess the sustained benefit.

Neurologic evaluation

Following recovery, a neurologist or developmental pediatrician should perform an examination that includes an evaluation for CNS injury using head CT scanning or MRI.

The incidence of hearing loss appears to be particularly high in patients with congenital diaphragmatic hernia (approximately 40% of infants). An automated hearing test should be performed prior to discharge.

The incidence of significant gastroesophageal reflux is very high in patients who survive congenital diaphragmatic hernia, and studies document an incidence of 45-85%.

The need for a diaphragmatic patch may be a significant predictor of gastroesophageal reflux. Severe reflux may result in chronic aspiration and is, therefore, aggressively treated.

Although most infants can be medically treated with H2-blockers or proton pump inhibitors in combination with a motility agent such as metoclopramide, surgical intervention is sometimes required.

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Transfer

Guidelines for ECMO consultation are available from the ELSO. Baseline criteria for ECMO consideration include evaluation for risk factors because of the invasive nature of the therapy and need for heparinization. Although criteria are center-specific, infants should generally be older than 34 weeks' gestation, have a weight greater than 2000 g, have no major intracranial hemorrhage on cranial sonography, have been on mechanical ventilator support for fewer than 10-14 days, and have no evidence for lethal congenital anomalies or inoperable cardiac disease.

Timing is always difficult, but referral and transfer should occur prior to refractory hypoxia. Early consultation and discussion with the ECMO center is strongly recommended.

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Prognosis

Overall reported survival varies among institutions. Remember that a single institution's results may look better than those provided by population-based studies because of case-selection biases. When all resources, including ECMO, are provided, reported survival rates range from 40-90%. The ELSO registry reports the ECMO survival rate at 52%.

As noted, survivors are at risk for significant long-term morbidity, including chronic lung disease, growth failure, gastroesophageal reflux, hearing loss, and neurodevelopmental delay. The risk appears to be highest in infants with severe lung disease (need for long term supplemental oxygen), need for patch closure of the diaphragm, and need for gastrostomy tube feeding.

Radiograph of a 1-day-old infant with a moderate-sized congenital diaphragmatic hernia (CDH). Note the air- and fluid-filled bowel loops in the left chest, the moderate shift of the mediastinum into the right chest, and the position of the orogastric tube.